Multiple Meningiomata in Cowden Syndrome

Abstract

Cowden syndrome (CS) is a rare, congenital disease with associated cancers, but in a neurosurgical context is typically considered part of Lhermitte-Duclos. This misrepresentation is the likely cause of under-diagnosis. Furthermore, the presence of meningiomata has been described in CS patients but its absence as part of the condition’s major criteria suggest the correlation requires greater documentation. A 41-year-old woman with multiple cancers and a familial circumambience of CS was reviewed in clinic where multiple meningiomata were incidentally identified on MRI. Despite a lack of neurological impairment, and the general reluctance in treating the meningiomas of congenital disease with radiotherapy (RT), the patient underwent stereotactic radio-surgery (SRS) and at one-year post-procedure has reported no side effects or toxicity.

Coexisting lipomatous meningioma and glioblastoma in Cowden syndrome: A unique tumor association

“…Our report and literature review support previous claims about the inclusion of meningiomas within the CS consensus diagnostic criteria. 21,22,24 So far, however, these tumors were not included in any updates. Nor are head studies recommended in the current screening guidelines for CS.…”

“…LDD, which is a neoplastic‐hamartomatous overgrowth in the cerebellum, and vascular anomalies are the only well‐recognized neuroimaging abnormalities associated with this rare PTEN hamartoma tumor syndrome 3,15,22 . A revision of the published reports of intracranial tumors other than LDD in CS patients identified a total of just 25 cases, 1,3,4,7–24 all of which corresponded to primary brain tumors (Table 1). Meningiomas were the prevailing ones and were found in 19 patients, 3,4,7,8,10,11,13–18,20–22,24 followed by pituitary adenomas, which occurred in three patients 13,19,23 .…”

“…A revision of the published reports of intracranial tumors other than LDD in CS patients identified a total of just 25 cases, 1,3,4,7–24 all of which corresponded to primary brain tumors (Table 1). Meningiomas were the prevailing ones and were found in 19 patients, 3,4,7,8,10,11,13–18,20–22,24 followed by pituitary adenomas, which occurred in three patients 13,19,23 . The remaining cases corresponded to two glioblastomas, which occurred in two males from the same family, 1 a medulloblastoma in a child, 9 and a pineal tumor in a young adult 12 .…”

“…A thorough literature review of CS patients' intracranial tumors other than LDD, despite the fact that this condition also exists in some cases concurrently, is also presented (Table 1). 1,3,4,[7][8][9][10][11][12][13][14][15][16][17][18][19][20][21][22][23][24] Our major aim is to raise awareness about the association between CS and other types of primary brain tumors.…”

“…A thorough literature review of CS patients' intracranial tumors other than LDD, despite the fact that this condition also exists in some cases concurrently, is also presented (Table 1). 1,3,4,7–24 Our major aim is to raise awareness about the association between CS and other types of primary brain tumors. We hope this knowledge will contribute to improving the management and cancer screening of CS patients.…”